These are some illustrations from a talk given to our cystic fibrosis group on April 24, 1998. The presentation will need some revising for the Web, but here it is for now:
This is a review of published literature that reports spinal bone density in either T or Z scores. In young adults the T scores are the same as Z scores so I substituted them.
| Number | Subjects | Z-score |
|---|---|---|
| 11 | Adult lung tx | -2.50 |
| 20 | adult lung tx | -2.14 |
| 16 | adults | -1.40 |
| 10 | adults | -1.47 |
| 70 | adults | -2.40 |
| 22 | adults | -2.80 |
| 49 | adults and children | -1.74 |
| 31 | adults and children | -1.00 |
| 62 | children | -1.30 |
| 28 | girls QCT | -0.31 |
| 29 | boys QCT | -0.97 |
FACTORS THAT COULD LEAD TO BONE DISEASE in patients with cystic fibrosis Malabsorption of vitamin D of calcium (.118 in normals .090 CF without enzymes .110 CF with enzymes) of general nutrients Low Vitamin D levels also depends on sun exposure measure 25 OH instead of 1,25(OH)2 unless: renal failure ketoconazole (can reduce 1,25) unexplained calcium abnormalities Hypogonadism high incidence in adult patients usually hypogonadotrophic Inactivity Respiratory acidosis Cytokines from chronic infections
TREATMENT OF BONE DISEASE in patients with cystic fibrosis Malabsorption Nutritional support Especially calcium Try calcium citrate Low Vitamin D levels Replace with 800-1000 units/day or more follow 25 OH vitamin D levels Hypogonadism Give teens and women estrogen if needed Avoid depo-provera Give males testosterone Inactivity Encourage exercise Respiratory acidosis - treat Cytokines from chronic infections - treat Use corticosteroids only when necessary In some cases may use calcitonin to mitigate effects But there MUST be adequate calcium/vitamin D Consider growth hormone
WHY TO AVOID BISPHOSPHONATES in patients with cystic fibrosis Etidronate (Didronel) Pamidronate (Aredia) Alendronate (Fosamax) May cause osteomalacia Patients with C.F. already prone to this May inhibit primary modeling of growing bone Safety in children not established only reported in a few cases should do bone biopsies if done on research basis Half-life in bone is >10 years! Poor absorption in normals probably worse in C.F.
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